Executive Functioning Mediates the Relationship Between Pain Coping and Quality of Life in Youth With Sickle Cell Disease

Ludwig, Natasha N; Sil, Soumitri; Khowaja, Meena; Cohen, Lindsey L.; Dampier, Carlton

doi:10.1093/jpepsy/jsy057

Cited by 34 publications

(25 citation statements)

References 48 publications

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“…These neurocognitive impairments are considered functionally and clinically significant as they can impact a person’s quality of life (Allen et al , ; Hardy et al , ; Ludwig et al , ) and vocational attainments (Sanger et al , ). In our sample, 31% repeated a grade and 81% received educational support at school, which indicates that most adolescents received supports.…”

Section: Discussionmentioning

confidence: 99%

Association between hydroxycarbamide exposure and neurocognitive function in adolescents with sickle cell disease

et al. 2020

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Summary Patients with sickle cell disease (SCD) are at increased risk for neurocognitive impairments. While disease‐modifying treatment, such as hydroxycarbamide (hydroxyurea), may decrease this risk, it has not been systematically investigated in children with SCD. We screened neurocognitive functioning in 103 adolescents with SCD (16–17 years, 50% female) and compared outcomes between patients with a history of exposure to hydroxycarbamide (n = 12 HbSC/HbSβ+ thalassaemia; n = 52 HbSS/HbSβ0 thalassaemia) and those never treated with hydroxycarbamide (n = 31 HbSC/HbSβ+ thalassaemia; n = 8 HbSS/HbSβ0 thalassaemia). Demographic distributions were similar between the groups. After adjusting for socioeconomic status, the hydroxycarbamide group had significantly higher scores on nonverbal IQ (HbSC/HbSβ thalassaemia: P = 0·036, effect size [d] = 0·65), reaction speed (HbSS/HbSβ0 thalassaemia: P = 0·002, d = 1·70), sustained attention (HbSS/HbSβ0 thalassaemia: P = 0·014, d = 1·30), working memory (HbSC/HbSβ+ thalassaemia: P = 0·034, d = 0·71) and verbal memory (HbSC/HbSβ+ thalassaemia: P = 0·038, d = 0·84) when compared to those who did not receive hydroxycarbamide. In patients with HbSS/HbSβ0 thalassaemia, longer treatment duration with hydroxycarbamide was associated with better verbal memory (P = 0·009) and reading (P = 0·002). Markers of hydroxycarbamide effect, including higher fetal haemoglobin (HbF), higher mean corpuscular volume (MCV) and lower white blood cell count (WBC), were associated with better verbal fluency (HbF: P = 0·014, MCV: P = 0·006, WBC: P = 0·047) and reading (MCV: P = 0·021, WBC: P = 0·037). Cognitive impairment may be mitigated by exposure to hydroxycarbamide in adolescents with SCD.

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Section: Discussionmentioning

confidence: 99%

Association between hydroxycarbamide exposure and neurocognitive function in adolescents with sickle cell disease

et al. 2020

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“…In our study children narrated how pain limited them from attending school as well as participating in play activities which negatively affected their quality of life. Ludwig et al found that executive functioning is an important factor in understanding the relationship between pain coping and HRQoL in youth with SCD [24].…”

Section: Discussionmentioning

confidence: 99%

Health-related quality of life of adolescents with sickle cell disease in sub-Saharan Africa: a cross-sectional study

et al. 2019

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Background Sickle cell disease (SCD) is a chronic hematologic disease associated with increased morbidity and mortality. Hemoglobinopathies are the most prevalent genetic disease globally, and SCD is estimated to affect 0.7% of Ugandan. The disease may adversely impact on the quality of life of sickle cell patients. This study aimed to evaluate the health related quality of life (HRoL) of adolescents with SCD. Methods This was a mixed-methods study of adolescents with sickle cell disease and their caretakers living in Kampala city, Uganda. All children aged 8–17 years with homozygous sickle cell disease attending the sickle cell clinic at Mulago Hospital during the study period were included in this study. Participants completed the PedsQL™ generic core scales parent-proxy and child self-report questionnaire during a routine clinic visit. HRQoL was the primary outcome measured. Socio-demographics and disease related data were obtained through personal interview with caretakers and reviewing patients’ medical records. Mean scores were used for HRQoL and linear regression for associated factors. Results Of the 140 adolescents with SCD included in the study, 40% were male. A total of 95 adolescents (68%) were between the age of 8–12 years with a mean age of 14.25 years. The physical function was assessed slightly higher by adolescents with a mean score of57.5 ± 20.3 compare to caretakers with 52.8 ± 22.1( p < 0.001). As assessed by caretakers, physical HRQoL scores were negatively associated with pain about-10.02 CI [− 19.22, − 0.81]( p = 0.033), whereas it was positively associated with Pneumococcal vaccine with the score of 28.43 CI [16.78,40.09]( p < 0.001) as assessed by adolescents and 31.37CI [22.22,40.51](p < 0.001) by caretakers. Pneumococcal vaccination impacted positively the psychosocial functioning with a score of 8.67CI [1.51,15.84] ( p = 0.018) as assessed by children and 15.94 CI [5.50,26.38]( p = 0.003) as assessed by the caretakers. Conclusions This study highlighted that pain was negatively associated with both physical and psychosocial functioning; whereas getting Pneumococcal vaccine was positively associated with both physical and psychosocial functioning as reported by children and caretakers.

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“…SCD-related medical complications and associated treatments have also been shown to contribute to poorer health-related quality of life (HRQOL), as have greater disease severity (Panepinto, O Mahar, DeBaun, Rennie, & Scott, 2004) and pain (Dampier et al, 2010;Ludwig, Sil, Khowaja, Cohen, & Dampier, 2018;Schlenz, Schatz, McClellan, & Roberts, 2012).…”

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confidence: 99%

Brief Screening Measures Identify Risk for Psychological Difficulties Among Children with Sickle Cell Disease

Hood

Reife²,

King

et al. 2019

J Clin Psychol Med Settings

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Children with sickle cell disease (SCD) experience disproportionately high rates of psychological problems. Our goal was to examine the clinical utility of psychological screening measures to identify children with such problems in medical settings. Caregivers completed screening measures assessing social-emotional problems, ADHD symptoms, executive dysfunction, and health-related quality of life (HRQOL) for children with SCD (receiving either chronic blood transfusion or hydroxyurea) and their siblings. Our findings demonstrated that screening measures identified clinically elevated symptoms in children with SCD that had not been previously reported. Scores for siblings were for the most part in the normal range. The number of days hospitalized (but not cerebral infarct status) predicted higher scores, emphasizing the challenges associated with SCD complications. Overall, our findings support the notion that screening measures reduce the need for reliance on medical provider judgment for psychological referrals and increase equitability in access to services. Early identification resulting in early intervention has contributed substantially to improved psychological functioning in many contexts, and it is thus likely that such improvements would also be achieved in this uniquely vulnerable population.

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Executive Functioning Mediates the Relationship Between Pain Coping and Quality of Life in Youth With Sickle Cell Disease

Cited by 34 publications

References 48 publications

Association between hydroxycarbamide exposure and neurocognitive function in adolescents with sickle cell disease

Association between hydroxycarbamide exposure and neurocognitive function in adolescents with sickle cell disease

Health-related quality of life of adolescents with sickle cell disease in sub-Saharan Africa: a cross-sectional study

Brief Screening Measures Identify Risk for Psychological Difficulties Among Children with Sickle Cell Disease

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